瑞图西马布相关的常见可变免疫缺陷如肠道病变的组织学谱
Pari Jafari1, David Hakimian2, Maria Westerhoff3
1Department of Pathology, University of Chicago Medicine, Chicago, Illinois.
概括
利图西马布 (RTX) 可以引起一种类似于CVID的肠道病变,伴有腹和吸收不良. 组织病理学揭示了血细胞枯竭,小性缩和亡的增加,这表明B细胞受损.
科学领域:
- 胃肠病学 胃肠病学
- 免疫学 免疫学 免疫学
- 病理学 病理学 病理学
背景情况:
- 抗CD20单克隆抗体瑞图西马布 (RTX) 用于B细胞瘤和自身免疫性疾病.
- RTX越来越多地与模仿常见可变免疫缺陷 (CVID) 肠病的肠病相关,出现腹,吸收不良和低甘球蛋白血症.
研究的目的:
- 进行第一个专门的 RTX 相关的 CVID 类肠道病变的组织病理学评估.
- 来自RTX相关肠道病变患者的小肠和结肠样本中的关键组织学特征的特征.
主要方法:
- 系统评估小肠和/或结肠活检/切除22名患有RTX暴露和胃肠道症状的患者.
- 纳入标准:腹,体重减轻,或与当前/之前使用RTX和低血糖球蛋白血症的胃肠道症状.
- 分析的组织学特征包括血细胞密度,内皮质淋巴细胞瘤,状结构,密室亡和炎症.
主要成果:
- 小肠的主要发现:稀少/缺少自身膜等离子体细胞 (50%),内皮细胞淋巴细胞症 (60%),小性缩 (55%),增加了隐藏式亡体 (30%).
- 结肠的主要发现:血细胞稀少/缺少 (41.2%),密码亡体增加 (41.2%),活性炎症 (29.4%),内皮细胞瘤 (23.5%).
- 在2名患者中观察到杯状细胞损失;随访活检在很大程度上回顾了最初的发现,其中一个病例出现了改善.
结论:
- 与RTX相关的CVID类肠病的组织学谱包括血细胞枯竭,加剧的密室亡,小性缩和杯状细胞损失.
- 临床病理学发现表明,与RTX后的B细胞/血细胞损伤存在潜在联系.
- 病理学家应考虑在患有类似CVID肠道病变的组织学特征的患者中暴露RTX,即使是微妙的.
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